| Prevalence | 1-10 per 100,000 population 4 |
| Geographic variation | Higher prevalence in Asian, African, and Latin American populations |
| Age of onset | Mean 30-40 years (range: <10 to >70) |
| Gender distribution | Strong female predominance (F:M = 9:1) |
| Ethnicity | More common in non-European populations |
| Incidence | Approximately 0.5-4 per 100,000 person-years |
| HLA associations | HLA-DRB1*03:01 and HLA-DPB1*05:01 associated with increased risk |
| AQP4 polymorphisms | Certain haplotypes increase susceptibility |
| Familial cases | Rare but reported, suggesting shared genetic susceptibility |
| Location | Perivascular and pial astrocyte processes |
| Function | Water homeostasis, glutamate clearance, potassium buffering |
| Expression | Highest in optic nerve, spinal cord, hypothalamus, circumventricular organs |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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